Holt Oram maʻi pale. Maʻi pale o ka "lima-i-ka naau"

Holt maʻi pale - Oram - mea he maʻi nui me autosomal hooilina type, a ua wehewehe aku ma lesions o mau nā'ōewe. Ka mea, e kuleana no ka anatomical 'ole o ka naʻau a me ka iwi. Kēia maʻi ua kapaia he maʻi pale o ka "lima-i-naau."

He aha ka mea?

Kēia maʻi ua pū i hiki ma ka hooilina, ka mea hiehie loli iki malformations o ka lunaʻaoʻao a me congenital naʻau maʻi. He nui e ike i ka maʻi i loko o kona ulu ana. E hana i kēia, e lawe mai aaiaoe noiʻi. Lakou i hoʻokūpaʻa wale i kaʻike,, akā, no hoi, e wńnana i ka likelihood o ka maʻi i loko o na hanauna e hiki.

Ultrasound keia hookolokolo ana ma ka hāpai keiki e leie kakahiaka ka loaʻaʻana o ka haunaele ma skeletal ulu. I ka hōʻike hoailona o ka maʻi i mālama 'vnutriplodnoe scan unborn pēpē naʻau. I hookuu i ka maʻi pale o Holt - ua paa ka manao o ka pēpē i ka hānau hope Oram. Hana kupua mā ma ka skeletal 'ole ke akahele hookolokolo aku la ia ma ka cardiologist me ka oolea o ka maʻi hilinaʻi nui ma luna o ka cardiac hoike ana mai o ka maʻi pale.

Congenital mā o ka maʻi

Wehewehe aku ma abnormalities i loko o ka 'ole o ka forearm a me ka lima, i ikeia ma kekahiʻaoʻao a ma nā ma kahi hoʻokahi. Ke degere o ka underdevelopment a me ka lala deformation mea okoa: ka mea, o ke alo o ka ekolu phalanges ma kekahi manamana lima a pau loa ia ma kahi'ē ka wā nui manamanalima. Loa pinepine, keia hana me na manamana lima ma kona lima hema. Aia mau loa hihia, ka wā he mea hypoplasia o ke kahahńnai, a piha hoʻi i emi kāna mau mea. Loa emi paha e olelo: fused me kēlā me nā manamana lima, sternum deformation, hypoplasia o na clavicles a me ka'ūhā mua kōmi 'ana, curvature o ka spine. Skeletal loli mai i oweliweli i ka hoʻoweliweli 'ana i kanaka ola. Akā, 'aʻe' ana o ka iaea ooieoeiiaeuiiai hiki o ka loli ana o hui, kēia maʻi i ola pilikia.

About 85% o nā mea maʻi e ae mai congenital naʻau mai: hou ana o ka leki uea a me ka hamama aortic stenosis o ka pulmonary artery, he 'aʻe' ana i ka nohona kiwikā 'ole. Pinepine i loaʻa congenital atrial septal kina. He mea okoa ma ka oolea mai māhunehune perceptible a hiki i ka nui haumāna kina, i mea heʻilihune o ka naʻau ole.

Autosomal Hoʻohaku hooilina

Kēia maʻi ua wehewehe aku e:

• Ka pololei, EYI o ka maʻi, mai kekahi hanauna aku i kekahi.
• Inā ka'ōewe mea nui proyavlyaemostyu, i ka maʻi 'aʻole i skip kekahi hanauna, a Hahai I ka' ohana ma kēlā me kēia 'ohana.
• Ke'ōewe Ua hala aku mai ka makua i ke keiki. Ke koe o ka mua manawa ua noho hihia o ka maʻi i loko o ka 'ohana no ka i ka noho'na makaainana i ulu mutations, i hope i hoolilo mai i ka maʻi makua i ola keiki kakou nana.
• Ma ka hapanui o na hihia i ka maʻi ua pū i hiki ma kekahi makua laina.
• 'Aʻole a pau i loko o ka ohana, e loaa mai ai iaʻu i ka maʻi'ōewe: mamo ma olakino ohana mau lala i hanau ai me ka hoailona o ka maʻi.

• Inā he keiki ka makuwahine, a me ka makua kāne nō okoa, a hope versa, ua okoa kekahi makua kāne a me ka makuwahine, i ka na makua o na keiki ke loaa i ka maʻi.
• An autosomal Hoʻohaku pololei o ka noho ana, aole ia e hilinaʻi ma luna o kekahi kanaka e pili ana i ka kekahi hoohanau. Na kanaka a me na wahine e like loli maʻi.

atrial septal

Inā he mea he kina i loko o laila, ulu congenital naau maʻi, i hiki ke kaʻawale a hui me ka 'ē aʻe olelo i loko o ka naau. Atrial septal kina e hoʻomōhala ana i ka wā e wehe ae kamaʻilio ua hanaʻia ma waena o nā atriaʻelua: haʻalele a me ka pono. Inā he hoomanawanui i ka maʻi maʻi pale Holt - Oram, oia ka mea, he pokole o ka hanu, ohumu naʻau, mahuahua luhi a stunted physical? Acaeoey, he mea pallor o ka ili a me ka pinepine naehana nā mea palahēhē. Congenital naau maʻi, kena ae la ia e keia pathology ia lŘlŘ i loko o kahi o 5-15% o nā mea maʻi, a me ka wahine ia lŘlŘ palua like pinepine ma mua o nā kānaka.

Ka mea i ka fetus oiai i loko o ka opu, o loko o ka atrial septum hamama puka'ololaha. Ua 'oi aku maʻamau koko holo. Once ka pēpē ua hanau, i ka puka i loko o ka hapanui o nā pēpē i pani. 'Oiai, he mau manawa, i ka wa keia' ole e hana. Akā, me ka hookuuia ana o ke koko ma ka puka uuku ia i kekahi kanaka, aole ia i haha aku i ka anomaly, a i ole i hāʻupu aʻe i ka mea i kekahi. He ola mālie a elemakule. Oia hereditary abnormalities like atrial septal kina, hiki ia i likeʻole nui like 'ole, ke ano, a ua loaʻa i loko o nā wahi likeʻole o ka mea.

Atrial mā. ano

• Ke anomaly o ka iniiaiie paku o keʻano o ka'āno'ē? Acaeoey cushions a me IIeEeAIEUIeXAIIUE ma ka haʻahaʻa o kona mau kolu kokoke trikulyarnymi kiwikā. Lakou, ma ka huli, hiki e ino a me ka hikiʻole ke hana kona manao kuleana pili i. Aia nā hihia wā me ka hoʻohana 'ia ua hana me ka pono ole atrioventricular septum kiwikā.
• Ma atrial septal kina o kona haumānaʻaoʻao, a ua ike i ka luna paha i loko o ka wahi o ka'ololaha fossa. Ma keia hihia, i ke koko komo ka atrium akau, mai ka hema. Pinepine i kapa mau mā kiʻekiʻe a me ka mea, e alaʻe mai ka nele o ka loaʻa nui o ka'aʻaʻa i pohā. Akā, ka mea i hoopaa mai ai i ka loiloi a me ka anatomical patency o ke kina.

Symptoms o atrial mā

• Holt maʻi pale - Oram pololei e pili ana i ka makahiki o ka hoomanawanui, me ka nui o septal kina, ka nui o pulmonary vascular e kū'ē'ē mai. He nui nā mea maʻi i keia kina nana pono ola a me ka mai ole hoi hoohewa aku. Ka mea i hiki e hookuu i ke koko, mai haʻalele i ka pono ma muli o ke excessive physical exertion a me ka luhi,ʻaʻole ma muli o ia he kina. Ma ka hapanui o na, ua i lohe i ka'ōhumu aku ai naʻau. Kēia kekahi manawa ka hana me ka nui atrial septal kina.

• Kēia anomaly He pinepine ke kumu o ka prolapse o ka mitral kiwikā. Nā mea maʻi i kēia maʻi, ua pono halulu mea a clicks.
• Inā ke kina nui ka nui loa, he kanaka i ka tachycardia, pokole o ka hanu, naau kuapuʻu, ka wā i ke kino o ka mokuna o ia mai kaʻaoʻao'ākau. O oluolu, ka hoʻohana 'ana i kou mau manamana lima, ho oholo i ka pulsation o ka pulmonary artery.
• Secondary septal hewa i loko o pohihihi keia hihia, ka mea i lilo i kumu o ka naʻau ole. Ua lŘlŘ ma 3-5% o nā mea maʻi, ka hapanui i loko o na keiki ma lalo o hoʻokahi makahiki. Inā 'aʻole' ole iʻoki kino, ka nui o ka pilikia o ka make.

Atrial mā. kumu

Anomalies ana underdeveloped iniiaiie a kiʻekiʻe i pohā zndokardialnye wā rollers i loko o ke au o embryonic ? Acaeoey.

• Ka hanana ana o ke kina, ua pono i ka aaiaoe, physical,? Aneie a me ka infectious aʻe.
• 'āno'ē kūpono 'ana o ke keiki paku i loko o ka opu i ka nui loa o ka pilikia i loko o nāʻohana o kona mau hoahanau i maʻi congenital naau maʻi.
• Maʻi mea hoʻomaʻi, wehewehe aku ma kekahi wahine hapai hiki i atrial septal kina. Ua rubella, herpes, moa pox, syphilis.
• Ai 'a me ka waiʻona i hāpai keiki.
• Nā palapala 'ona i loko o ka? Iecaianoaaiiie, irradiation.
• Complications aie i kaʻawahia, i kūpono i ka gestation ke keiki ka compromised.

Atrial septal mā. Mea Maluna

I ka manawa, mai ke kolu a hiki i ka hebedoma walu o ka hāpai keiki i loko o ka opu i manaʻo ma ka fetal naau. Inā i kāpili paku ma waena o nā halves akau a me ka hema o ka naau, ua hiki aku i ka mea i loaʻa nā mā ma ka atrial septum, a oko a ma ka nui, ka helu a me kaʻoihana o ka puka. Mea Maluna o ka congenital naʻau maʻi, ua like penei:

• N mā i wehewehe aku e nui Ana: ekolu i elima mau kenimika. o ka wahi o kona IIeEeAIEUAA ka malalo o ka septum, i kahi ma luna o ka atrioventricular kiwikā, e like ma luna o ia mau mea. Ka mea, e mai i ka lalo lihi.
• Secondary mā i hanaʻia ma muli o ka underdevelopment o ka haumāna i pohā. Ka mea, he poe uuku ma ka nui a ma kona kikowaena hapa paha i loko o ka waha o ka puha aa koko o ka wahi. Ua hana ia mā septum a'āno'ē haule i loko o ka atrium akau Pulmonary aa koko i hui. Ma keia hihia, no ka interatrial septum ua paa i ka lalo.
• Aia mau hihia, i ka wa o ka iniiaiie a me ka kiʻekiʻe mā ka nanaia mai ka poʻe.
• Interatrial septum paha hoi ma kahi ee. Kēia hualoaʻa ma ka pono ole, pau atrium, i ua kapaia ekolu-keʻena naʻau.

Congenital naʻau maʻi. lapaʻau '

Inā ke keiki ua mahuʻi mua o ka maʻi pono kaʻenaʻena kapa cardiologist. Hoʻomalu hoʻokolokolo ai na symptoms, assesses ke ano a me ka puʻe pana, ka moku'āina o nā mea a pau nā loko, a nenoai. Ma hope o inspecting i ke kamaiki ua paa electrocardiogram, ultrasound, phonocardiogram, X-Ray o ka naau. Ma ka hanana o ka nui kino o ka maʻiʻike, o congenital malformations ua complemented ma kekahi anamanaʻo - cardiac catheterization ka wā injected i loko o kona luaʻimi.

Loa pinepine, na makua pēpē poe i ke kauka i ole hōʻike i kēia pilikia maʻi, i ka wa o ka expectant makuahine ma ka hāpai keiki i malama i loko ona. Ke kumu o ka congenital naau maʻi hiki ia i:

• Low nui o ka 'inikua professionalism.
• Congenital maʻi i ole mau hiki ke diagnose i ka manawa ku i ka 'o ka' ole o nā ipu naau a me ke koko o ka fetus.
• Hemahema o ka 'inikua lako a pan.

Nā kumu o ka congenital naʻau mā

Congenital naau mā i na kamalii uuku e ku pono i ka anatomical mā o ka naau a me kona kiwikā a me nā ipu koko, i kānāwai i loko o ka opu. Ma mua, ka mea iʻike makaʻole e, a ua hiki ia i hookahuli ai i ke keiki koke ma hope o ka hanau ana. Ka maʻi puʻuwai - o ka loa e like me ka hanau mā i loko o nā pēpē i, a me ka mooolelo no ma kahi o 30%. Ka mea,ʻo ka loa pinepine kumu o ka make. Kumu o atrial septal kina:

• Neʻe i hāpai keiki, mea hoʻomaʻi maʻi e like me rubella,ʻo Waimānalo a me na mea e ae.
• Ke aaiaoe mutation, kena ae la ma ka hoʻohana 'ana i ka wā e hiki mai makuwahine o ai', ka waiʻona, nicotine.
• Ke Kula Kaiapuni 'abnormalities o chromosome e puhi ia o na makua.
• Waiū i ionizing pāhawewe.
• Deficiency o ke kumumea oihana mua a me nā wikamina i loko o kaʻai o ka wahine hapai.
• Waiwai o ka lapaʻauʻia ma luna o ka fetus.

Symptoms o congenital naau maʻi

Hanau keiki me ka kāna hana iʻokoʻa. Ka mea,ʻo ka meaʻili, na lehelehe, i ka pepeiao i ka bluish a uliuli waihoʻoluʻu. I kekahi manawa, ma ka mea ku ole, bebeʻili pale a me ka'ūhā, ua anu. Keia ua kapa keʻokeʻo kina nā pēpē. Aia mau hihia, i ka wa e hoolohe ana i ka akamai maopopo lohe i ka'ōhumu aku ai naʻau.

Loa pinepine congenital hui, kēia maʻi imperceptibly koke ma hope o ka pēpē ka hānauʻia. No ka 10 makahiki, i ke keiki, e nana pono ola. Akā, laila, i ka maʻi, e hoomaka i ka hoike i ke ano o ka uliuli i loko o ka maka a me blanching o ka ili. Lilo kekahi 'deviations ma physical kūpono' ana, ma laila e e pōkole o ka hanu.